Abstract:
:Activation and regulation of the cascade systems of the blood (the complement system, the coagulation/contact activation/kallikrein system, and the fibrinolytic system) occurs via activation of zymogen molecules to specific active proteolytic enzymes. Despite the fact that the generated proteases are all present together in the blood, under physiological conditions, the activity of the generated proteases is controlled by endogenous protease inhibitors. Consequently, there is remarkable little crosstalk between the different systems in the fluid phase. This concept review article aims at identifying and describing conditions where the strict system-related control is circumvented. These include clinical settings where massive amounts of proteolytic enzymes are released from tissues, e.g., during pancreatitis or post-traumatic tissue damage, resulting in consumption of the natural substrates of the specific proteases and the available protease inhibitor. Another example of cascade system dysregulation is disseminated intravascular coagulation, with canonical activation of all cascade systems of the blood, also leading to specific substrate and protease inhibitor elimination. The present review explains basic concepts in protease biochemistry of importance to understand clinical conditions with extensive protease activation.
journal_name
Semin Immunopatholjournal_title
Seminars in immunopathologyauthors
Huber-Lang M,Ekdahl KN,Wiegner R,Fromell K,Nilsson Bdoi
10.1007/s00281-017-0646-9subject
Has Abstractpub_date
2018-01-01 00:00:00pages
87-102issue
1eissn
1863-2297issn
1863-2300pii
10.1007/s00281-017-0646-9journal_volume
40pub_type
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